AORTIC VALVULAR STENOSIS WITH COARCTATIONOF THE AORTA

WITH SPECIAL REFERENCE TO THE DEVELOPMENT OF AORTIC STENOSISUPON CONGENITAL BICUSPID VALVES

BY

D. E. SMITH* AND M. B. MATTHEWS

From the Cardiac Department, St. Thomas's HospitalReceived August 21, 1954

The combination of coarctation of the aorta and aortic valvular stenosis is not well known,although the association of coarctation with aortic incompetence is generally recognized and usuallyattributed to an incompetent bicuspid aortic valve. Abbot (1928), in her review of coarctation,did not discuss its association with aortic valvular stenosis. However, she reported four cases ofsub-aortic stenosis occurring with coarctation (an incidence of 2%), and Taussig (1947) regardsthis combination as occurring so frequently as to constitute a clinical entity. Wood (1950) statesbriefly that with coarctation " aortic or sub-aortic stenosis is by no means rare." Reifenstein etal. (1947) in their series of 104 post-mortem cases of coarctation record 11 with " calcific aorticstenosis " (each of which they regard as being due to rheumatic disease), but they do not enlargeupon this association. Recently Jacobson et al. (1953) have collected 14 reported cases of" calcific aortic stenosis " complicating coarctation, and add one further case; they commenton the surprising rarity of bicuspid aortic valves, which they found in only 3 of 14 necropsycases [one (Gilbert et al., 1950) lacks post-mortem confirmation], and on the rarity of calcificaortic valves.

Five cases of aortic stenosis complicating coarctation have recently come to our attention.This has prompted a review, from which we have collected 24 cases with necropsy confirmation ofthis combination.

CLINICAL MATERIAL(A) Present Series. Five new cases of coarctation of the aorta with aortic valvular stenosis

are reported; three of these have necropsy confirmation, the other two are living.

Case 1. Man, aged 43. A murmur was discovered on routine physical examination at school at8 years. He was refused life insurance at 17 years, and high blood pressure was found at 22 years. For twoyears before admission he had complained of giddiness, sweating, and nervousness. On examination therewas a systolic thrill and harsh systolic murmur in the second right intercostal space. The second aorticsound was normal. No diastolic aortic murmur was heard, but a mid-diastolic murmur was audible at theapex. A systolic murmur was heard over the back. The radial pulses were equal and the femoral pulseswere weak and delayed. The blood pressure was 170/100 in both arms. A cardiogram showed leftventricular hypertrophy and auricular fibrillation. X-ray and screening revealed marked left ventricularenlargement, prominent ascending aorta, extensive rib-notching, but no left auricular enlargement. Thepatient died of a cerebral vascular accident with a left hemiplegia.

Necropsy. Severe coarctation immediately distal to the origin of the left subclavian artery, whichadmitted only a surgical needle. There was a marked collateral circulation. The pericardium was unusually

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thin. Both auricles were free from clot. The aortic valve was stenosed, with large calcified irregular cusps,and the left ventricle was hypertrophied. There was no mitral stenosis. There was a large embolus at thebifurcation of the right common carotid artery, apparently blocking both internal and external branches.Another embolus occluded the left internal carotid. There was infarction of the posterior part of the rightinternal capsule and of some of the right occipital lobe. The left kidney had numerous infarcts. A sectionof the ileum was gangrenous.

Microscopical sections of the aortic and mitral valves were not made. There was thickening of thepericardium over the auricle, and of the endocardial lining. The ventricle showed thickening of the endo-cardium and atheroma of the coronary arteries. Section of the heart muscle through the auriculo-ventricularjunction showed perivascular fibrosis of the small vessels with persisting cellular elements.

Final diagnosis. Coarctation of the aorta, calcific aortic stenosis, multiple emboli, the source of whichwas not found, and possible rheumatic heart disease.

Case 2 (Dr. Goodwin's case). Woman, aged 18 years. She complained of dyspnca on effort for sixmonths. There was a systolic thrill and murmur, maximal in the second right intercostal space. The apexbeat was heaving and of left ventricular type. The blood pressure was 190/120 in both arms. The femoralpulses were feeble and delayed. A cardiogram showed marked left ventricular hypertrophy. X-ray andscreening showed left ventricular enlargement, a small aorta, and rib-notching. The patient died four hoursafter a resection of the coarctation from hemorrhage at the suture line.

Necropsy. Bicuspid aortic valve, cusps thickened and orifice stenosed. Marked left ventricular hyper-trophy. Microscopic sections from the various valves, valve rings, and left ventricle showed no signs ofcarditis.

Final diagnosis. Coarctation of the aorta and congenital bicuspid aortic valve with valvular stenosis.Case 3 (Dr. Bret's case). Man, aged 18 years. A murmur was first discovered at eight years. At

12 years coarctation of the aorta was diagnosed. Dyspncea had been noted from the age of ten. There hadbeen two episodes of hamoptysis within two years of death. On examination there was an aortic thrilland -murmur, maximal in the second right intercostal space; the murmur was not heard in the neck. Therewas no aortic diastolic murmur, and no apical murmurs. The blood pressure was 125/? in both arms.The radial pulses were equal, the femoral pulses absent. A cardiogram showed left ventricular hypertrophy.X-ray and screening showed a prominent ascending aorta, and left ventricular hypertrophy. There was norib erosion. The patient died at the time of aortography.

Post-mortem findings. Coarctation of the aorta close to the subclavian artery, admitting only a stilette.In addition there was stenosis of the aortic orifice with a bicuspid aortic valve and dilatation of the ascendingaorta. There were no other cardiac malformations. No microscopical sections were made.

Final diagnosis. Coarctation of the aorta and congenital bicuspid aortic valve with valvular stenosis.Case 4. Man, aged 52. Rheumatic fever at 6 years. Increasing effort dyspncea for a year before

admission, culminating in attacks of paroxysmal dyspncea. On examination the apex beat was in the sixthspace at the anterior axillary line. There was a harsh systolic murmur over the precordium, loudest in thesecond right interspace, and propagated to the neck. There was also an aortic diastolic murmur and amitral diastolic murmur. The femoral pulses were strong but collateral circulation apparent. The bloodpressure was 290/90 on admission and 190/70 four days later. There were basal crepitations. A cardio-gram showed complete heart block and right bundle branch block. On X-ray and screening there wascardiac enlargement, pulmonary congestion, rib notching, and a calcified aortic valve. A Wassermannreaction was negative. The patient responded to treatment for heart failure and was discharged.

Clinical diagnosis. Coarctation of the aorta, calcific aortic stenosis and aortic insufficiency, congestivefailure, and ? mitral stenosis.

Case 5 (Dr. Goodwin's case). Man, aged 55 years. He had been aware of heart disease since childhood,but there was no history of rheumatic fever or chorea. He had always noted shortness of breath on exertion,which had increased since pneumonia three years previously. The admission to hospital was due to a furtherattack of pneumonia. On examination there was a systolic murmur, loudest in the aortic area, and a softaortic diastolic murmur. There was a moderate systolic murmur and a soft rumbling mid-diastolic murmurat the apex. The blood pressure was 170/60 in both arms, and 110/50 in the legs. A collateral circulationwas evident. There were basal crepitations. A cardiogram showed marked left ventricular hyp-rtrophy andintra-cardiac block. On X-ray and screening there was gross left ventricular enlargement, calcification ofthe aortic valve, and marked dilatation of the ascending aorta and arch. The patient was discharged aftertreatment for the congestive cardiac failure.

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Clinical diagnosis. Coarctation of the aorta, calcific aortic stenosis and incompetence, congestive failure,and ? mitral stenosis.

(B) Cases reported by Other Authors. With Necropsy. Table I records 24 cases of coarctationaccompanied by aortic stenosis as the only valvular lesion. In many of the later cases the micro-scopic findings have been recorded by the original observers in some detail, and therefore permit anopinion as to the etiology of the valve lesion; in the earlier cases there was no reason to questionthe conclusions of the original authors. Only one (Case 25) had an "infantile " rather than anadult type of coarctation.

Without Necropsy. Several cases of this syndrome have been reported, without necropsy control.Grishman et al. (1947) report one with aortic stenosis associated with coarctation (their Case 6)and two with other anomalies of the aortic arch (their Cases 2 and 5). Gilbert et al. (1950) reportthe case of a man, aged 52, with the history of an aortic valve lesion at least since the age of thirty:there was no history of rheumatic fever, and the clinical evidence of coarctation was unequivocal;aortic stenosis was diagnosed on the basis of a systolic murmur and thrill, and calcification of theaortic valve was seen radiologically. It is interesting to note that the blood pressure had been highat the age of 12 (160/95), when the patient was refused life insurance; in later life it was recorded asrelatively normal in the arms.

Marquis (1954) has recently reported two cases of this syndrome.Two of our cases are based on clinical findings; each had calcification of the aortic valve on

screening.

DISCUSSIONAs would be anticipated in coarctation (and also in aortic valvular disease), the males outnumber

the females, 4 to 1. This is the proportion that Abbott (1928) found in her 200 cases of coarctation.The average age at death was 38 years. Reifenstein et al. (1947), in their review of 104 autopsied

cases of coarctation, found the average age at death to be 35 years. They considered that thosepatients with complicating valvular disease usually died of congestive failure, and at a later age

than those who died of other cardiovascular complications (aortic rupture, cerebral hemorrhage,and bacterial endocarditis or aortitis). The natural history of these patients appeared to be thatof valvular disease rather than of coarctation.

In the present series bicuspid aortic valves were recorded in 20 of 27 cases, an incidence of74 per cent. This is almost three times the incidence found by Abbott (1928) (25%), and nearlytwice that found by Reifenstein et al. (42%) in their respective series of cases of coarctation.

In the earlier cases it is not possible to determine whether the bicuspid valves were acquired or

congenital. Of the six where adequate microscopical details are given, five had a congenitalbicuspid valve. In each case aortic valvular stenosis had developed without any gross or micro-scopical evidence of rheumatic heart disease. Calcification was recorded in three of these. Inone case with adequate microscopical detail calcific aortic stenosis had developed on an acquiredbicuspid valve. Calcification was described in 14 cases.

It is generally accepted that it may be difficult to decide, even with detailed microscopical examina-tion, whether rheumatic valvulitis has played a part in producing stenosis of a bicuspid aortic valve.In this respect a report that microscopical evidence of rheumatism was absent was consideredparticularly significant.

It appears that the association of aortic valvular stenosis with coarctation of the aorta has beenlargely overlooked. Abbott (1928), in her review of 200 cases, reported only two as having aorticvalvular stenosis. A re-examination of the original reports upon which her review was based showsthat in a further11 cases aortic valvular stenosis was described, making an incidence of 65 per cent

in her series.A bicuspid aortic valve appears to be about twice as common when aortic valvular stenosis

complicates coarctation of the aorta as in a general series of coarctation.

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TABLE IPOST-MORTEM CASES OF AORTIC VALVULAR STENOSIS COMPLICATING COARCTATION OF THE AORTA *

201

Aortic valveCase Author Sex Age RemarksNo. Orifice Cusps

Craigie, Case 9 (1841)Peacock, Case 24 (1860)Peacock, Case 27 (1860)

Peacock, Case 29 (1860)Barie, Case 58 (1886)

Barie, Case 78 (1886)

Barie, Case 70 (1886)Bonnet, Case 14 (1903)

Bonnet, Case 25 (1903)

Fawcett, Case 3 (1905)Fawcett, Case 10 (1905)

Hamilton and Abbott (1920)

Abbott (1928) quotes Reinitz (1902)

Rosler (1928)Bode and Knop (1928)4

Hein (1931)

Bishop and Trubek (1936)

Benkwitz and Hunter (1937)

Ingham (1939)

20 Gitlow and Sommer (1940)

Lewis (1945)

Clark and Firminger (1949)

Jacobson et al., Case 3 (1953)

Brown (1934)

Present series, Case 1Present series, Case 2

Present series, Case 3

MFM

MF

M

MM

I?

FF

M

M

MM

M

M

M

M

M

M

M

M

F

MF

M

272014

3219

65

3825

c. 4534

14

34

4554

60

45

37

50

45

38

32

38

3days

4318

18

Constricted *tContractedSomewhatnarrowNarrowVery stenosed

Considerablestenosis

Stenosed andincompetent

StenosedStenosed

Stenosed andincompetent

Stenosed

Severe stenosisStenosed

Stenosed

Narrow slit

Stenosis

Severe stenosis

Severe stenosis

Stenosed

Stenosed

Stenosed

Severe stenosis

Severe stenosisSlight stenosis

Severe stenosis

CalcifiedBicuspidBicuspid**

ImmovableBicuspid

Bicuspid; calci-fied

BicuspidBicuspid

BiscupidBony cretaceousvegetations

Bicuspid* *

Bicuspid**; calci-fied

BicuspidBicuspid**; calci-fied

Calcified;" valveincomplete "

Bicuspid; calci-fied

Bicuspid (prob-ably congenital);calcified

Congenitalbicuspid

Bicuspid (prob-ably congenital);calcified

Congenitalbicuspid;calcified

Congenitalbicuspid;calcified

Bicuspid* *; calci-fiedA fibrousdiaphragm

CalcifiedCongenitalbicuspid

Bicuspid**

Patent ductus arteriosus

Widely patent ductusarteriosus

Cusps partially fusedAnterior cusp rigid andcalcified

Aortic aneurysm distalto coarctation

Patent ductus arteriosusMiniature supernu-merary cusp i in. abovevalve; bacterial endo-carditis

Sub-aortic stenosis;dissecting aneurysm ofaorta: adherent peri-cardium

Ductus partially obli-terated. Microscopicalexamination suggestedinflammatory fusion ofaortic cusps

Sub-aortic stenosis;calcified

Microscopically noevidence of inflamma-tionMicroscopically noevidence of syphilis orrheumatic heart diseaseMicroscopically noevidence of an acquiredunion between thecuspsMicroscopically noevidence of fibrosis orinflammation

Microscopically noendocarditis. Congen-ital stenosis

Microscopically norheumatic heart disease

123

45

6

78

9

1011

12

13

1415

16

17

18

19

21

22

23

24

2526

27

* In each case the aortic lesion was the only valvular lesion reported. The opinions recorded are those of theoriginal authors. ** Probably congenital.

t Also reported by Peacock (1860, Case 9).1 This case was more fully reported by Weber and Knop (1929).P

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The AEtiology ofAortic Stenosis associated with Coarctation of the Aorta. Brown (1934) describedthe case of an infant dying three days after birth who had congenital aortic valvular stenosis, coarcta-tion of the aorta, a widely patent ductus arteriosus, and a patent foramen ovale. " There was afibrous diaphragm at the site of the aortic valves-this was thick and hard and was pierced by aminute opening. The cusps could not be differentiated." There was no evidence that the stenosisof the aortic orifice was due to a faetal endocarditis. Brown concludes: " A search of the literaturehas failed to reveal one (case) in which orificial stenosis of the aorta was combined with isthmusstenosis." In this he presumably refers to congenital orificial stenosis.

Brown's appears to be the only case in which congenital aortic stenosis was associated withcoarctation of the aorta. It is apparent from the above autopsy reports (Table I) that aortic valvularstenosis, when complicating coarctation of the aorta, is an acquired lesion, though this does notexclude the possibility of an underlying malformation. However, Bishop and Trubeck (1934)conclude that the presence of an associated congenital cardiovascular anomaly, such as coarctationof the aorta, cannot be taken as presumptive evidence that the calcified aortic stenosis has developedon a malformed valve. Fawcett (1905), in a review of 18 cases of coarctation, stated that "Theconstant strain to which the aortic valves are subjected leads in the majority of cases to a chronicinflammatory change with the result that the valves become thickened, or adherent, or the orificestenosed." He made no mention of bicuspid aortic valves, so that it must be assumed that he wasreferring to changes in a previously normal valve. Reifenstein et al. (1947) concluded that their 11cases of aortic stenosis associated with coarctation were all attributable to rheumatic heartdisease.

The frequent association of coarctation of the aorta with congenital bicuspid aortic valves hasalready been noted. Abbott reported a further four, and Reifenstein et al. three cases of apparentlyacquired bicuspid valves. Conversely, Lewis and Grant (1923) found that coarctation was thecommonest malformation associated with undoubted congenital bicuspid aortic valves. A bicuspidaortic valve occurring with coarctation is, therefore, almost always congenital. Jacobson et al.(1953) commented on the rarity of bicuspid valves in this syndrome, but in the present series bicuspidaortic valves were found in 20 of 27 cases (Table II).

TABLE 11INCIDENCE OF BiCUSPID AORTIC VALVES IN COARCTATION OF THE AORTA AND AORTIC VALVULAR STENOSIS

Total number of cases of coarctation and aortic stenosis: 27Bicuspid valve (1) inflammatory 1

(2) congenital 5(3) probably congenital 7(4) undetermined 7

20Total number of aortic valves calcified .. .. 14Number of bicuspid aortic valves calcified .. .. 9

Bicuspid Aortic Valve as a Basisof Aortic Stenosis. The first worker to remark on the fact thatbicuspid semilunar valves are prone to disease appears to have been Paget (1844): "It has never,I believe, been noticed (though there need be no doubt about the fact) that in the majority of casesin which only two valves have been found in the aorta or pulmonary artery, these valves have beendiseased and often extremely diseased." Peacock (1851) was among the first to be aware that thehigh pressure to which the aortic valve is exposed makes it vulnerable to disease. "We knowthat in after-life the variable pressure of the blood in the arterial system is a fruitful cause of diseasein the aortic valves."

These two concepts are of special application in coarctation, where hypertension is almostinvariably present and a congenital bicuspid valve common. Peacock also demonstrated clearlythat the congenital bicuspid aortic valve could become diseased, and either stenosed or incompetent:

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'... . the defect described (bicuspid aortic valve) is shown to be of congenital origin, at least in manyinstances, by its co-existence with other obvious malformations, and by its occurring in childrenwhich survive birth only a short period, and even in foetuses which have never breathed. . .(Peacock, 1886, pp. 131 and 132.)

" The forms of the defect (bicuspid valve) now described, though not necessarily interfering withthe functions of the heart, do, in a considerable number of cases lay the foundation of disease inafter-life." . . . " When the whole of the segments are fused together or when two only are united,the valves not infrequently become the seat of chronic inflammation, by which they are renderedthick and unyielding and often become extensively ossified, thus inducing, first obstruction to theflow of blood from the ventricle into the aorta, and then incompetency." (Peacock, 1886, p. 133.)

Earlier, in his Croonian lectures of 1857, Peacock had expressed views similar to the above,and added: " I believe, however, that when the symptoms and signs of uncomp'icated aortic valvulardisease manifest themselves in persons who have never had rheumatic fever or other serious illness,... we shall generally be correct in assigning its probable production to malformation of the valves."(Peacock, 1857, p. 31.) From this it is clear that Peacock believed that the bicuspid aorticvalve frequently becomes stenosed without superimposed rheumatic valvulitis.

Many workers since Peacock have noted that congenital bicuspid valves usually show secondarychanges in the adult. Osler (1886) remarked: " The united cusps may have a perfectly normalappearance, but in adults they are almost invariably thickened and the seat of sclerotic or, insome cases, ulcerative changes . . . doubtless the strain on the fused curtains is more severethan upon normal cusps." He commented on the frequency of associated left ventricularhypertrophy.

Lewis and Grant (1923) noted that in congenital bicuspid valves thickenings were " the rulerather than the exception " and occur " as secondary processes in aortic valve margins which failclosely to adjust themselves when the valve shuts." Their investigations included detailed micro-scopy of bicuspid valves; they regarded these changes as due to faultily functioning valves and notto a rheumatic valvulitis.

Koletsky (1941), in a study of 18 cases of congenital bicuspid aortic valves concluded, " Thisanomaly also may pre-dispose to calcific sclerosis," and considered that the association of calcificsclerosis and a congenital bicuspid valve was not very rare. Although he does not discuss aorticstenosis in this communication, in one at least of his cases (Case 13) calcific aortic stenosis wassuperimposed on a congenital bicuspid aortic valve, and there was no microscopic evidence ofrheumatic disease in sections of the heart, valve rings, and valves. He also states: " The inherenttendency of the congenital bicuspid valve to undergo degeneration and fibrosis has been pointed outfrequently and is attributed to the fact that abnormally developed cusps are composed of imperfector defective tissue. The degeneration and fibrosis are undoubtedly accelerated with age, and inolder persons, particularly those with an especial tendency to arteriosclerosis, would be prone tolead to the deposition of calcium."

In considering the general problem of calcific aortic stenosis, Karsner and Koletsky (1947)concluded that this lesion is almost always of rheumatic origin. Of 200 cases, only four were notassociated with rheumatic stigmata. These conclusions were based essentially on microscopicalfindings. However, Hall and Anderson (1943) have shown that, if the criteria of Gross et al. (1930)are valid for the diagnosis of rheumatic heart disease, the incidence of rheumatic fever is comparableto that of tuberculosis and infantile paralysis. On these grounds the conclusions of Karsner andKoletsky are open to criticism. Kiloh (1950) concluded that a congenital bicuspid valve may wellplay an important role in the development of calcific aortic stenosis. A similar conclusion isreached by Bailey et al. (1954).

Hall and Ichioka (1940) studied the etiology of 31 cases of nodular calcific aortic stenosis.They divided these cases into 19 with isolated aortic lesions, and 12 with combined aortic and mitrallesions. Using the standard microscopical criteria for rheumatic disease, they concluded that the

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changes in all the cases appear to have a common etiology and fulfil the criteria generally acceptedfor chronic rheumatic infections of the heart. The same criticism of these findings may be madeas were made of those of Karsner and Koletsky. In addition, it is of interest to note that in 8 ofthe 19 cases with isolated aortic valve disease bicuspid valves were found, but " strangely none wasfound in the mixed group."

It is apparent then, that aortic stenosis can develop on a congenital bicuspid valve independently

AFIG. 1.-Drawing (A) and X-ray (B) to show calcification in a congenital bicuspid aortic valve which

produced aortic stenosis, in a man, aged 60. It is one of Peacock's spacimans in St. Thomas'sHospital Museum (No. lA.1.28), and was originally reported by Bennett (1851), who describedthe obstruction to blood flow as being due to rigidity of the cusps. [A rudimentary third valvebetween the other two can be seen in the specimen and drawing; in Peacock (1886) there is an en-graving of the specimen (Plate VII, Fig. 3) where it has been exaggerated.]

A BFIG. 2.-Drawing (A) and X-ray (B), showing calcification in a congenital bicuspid aortic valve in a

man, aged 65, with characteristic signs of aortic stenosis. He died suddenly during treatment forleft ventricular failure. Note that the obstruction to blood flow is due to rigidity of the cusps;there is no commissural fusion. The left ventricle was markedly hypertrophied. (St. Stephen'sHospital, Case No. 36309.)

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of rheumatic inflammation. Reifenstein et al. (1947) and Wauchope (1928) have, however, sug-gested that the congenital bicuspid aortic valve is more prone to rheumatic disease than normally-formed cusps. From the necropsy reports in the present series we suggest that Peacock was correctin concluding that valvular aortic stenosis could arise from degeneration, usually with calcification,in a congenital bicuspid valve. The liability to such changes is increased by the abnormal mechanicalstresses of a malformed and malfunctioning valve, and the process hastened by the higher pressuresimposed by the coarctation. A similar interpretation of aortic incompetence in coarctation of theaorta with a bicuspid aortic valve is used by Bramwell and King (1942).

Induration and calcification of the cusps, rather than commissural fusion, may be the cause ofobstruction at the valve orifice in some cases. Fig. 1 and 2 show the degeneration and calcificationthat occurred in two congenital bicuspid valves. The first example is of interest in being one ofPeacock's own cases from the Museum at St. Thomas's Hospital, and shows extensive calcifica-tion in the bicuspid valve of a male, aged 60. The second is from a man of 65 who died of cardiacfailure from aortic stenosis. In neither valve was there evidence of an acquired fusion. In bothcases left ventricular hypertrophy demonstrated that there had been obstruction to blood flow atthe valve. In each the bicuspid valve occurred as an isolated anomaly.

We conclude, therefore, that aortic stenosis is quite common in coarctation of the aorta, andthat a congenital bicuspid valve is the common factor.

We suggest that these conclusions may have a wider implication in accounting for some cases ofisolated calcific stenosis of undetermined aetiology. It is possible that some of those patients whosebicuspid aortic valves are not revealed by subacute bacterial endocarditis may present in old agewith the signs of aortic stenosis as the first overt evidence of this malformation. Support is lent tothis conclusion by the fact that, in old age, aortic stenosis is commoner in men than women.

Often the calcified aortic valve can be seen to be bicuspid, but if the calcification and destructionof the valve are far advanced, as is so frequently the case, a correct morphological interpretation maybe impossible and the histological picture confused.

Note on Diagnosis. The clinical diagnosis of aortic stenosis in the presence of coarctation isimportant now that surgical treatment is possible. Uncomplicated coarctation of the aorta mayproduce systolic murmurs sufficiently loud to cause a thrill (Reifenstein et al., 1947), and the thrillmay disappear after a successful resection of the coarctation (Goodwin, 1954). If the murmur isobviously louder to the right of the sternum than to the left, the possibility of aortic valvular stenosisshould be considered.

If there are typical enlarged collateral blood vessels, and a weak and delayed or absent femoralpulse, the finding of a normal blood pressure in the arms is extremely suggestive of co-existing aorticstenosis. Bishop and Trubeck (1936) report the finding of a blood pressure of 110/70 in the upperextremities in a man of 45 with this combination (Case 18, Table I). In Cases 15 and 27 the bloodpressure in the arms was also normal. In the clinical case of Gilbert et al. (1950) already noted, theblood pressure in the arms had been raised at the age of 12 (165/95), but was normal 40 years later.

The brachial arterial pressure pulse may be helpful if it shows prolonged systolic ejection oranacrotism, as suggested by Grishman et al. (1947). The differentiation of valvular from sub-valvular stenosis is impossible, though calcification visible on X-ray is more suggestive of theformer. Evidence of left ventricular hypertrophy on X-ray screening, or from the electrocardio-gram is to be expected more frequently than in isolated coarctation.

SUMMARY AND CONCLUSIONSFive cases of aortic valvular stenosis combined with coarctation of the aorta are described. In

three there was necropsy control, and in the other two radiological evidence of calcification of theaortic valve. A further 24 similar reported cases with necropsy control are reviewed. Are-examination of the original reports upon which Abbott's (1928) review of 200 cases ofcoarctationwere based shows the incidence of aortic valvular stenosis to be 6 5 per cent.

Of the 27 cases with necropsies, 20 had a bicuspid aortic valve. In 5 of the 6 where microscopy

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was adequate, the absence of rheumatic stigmata was taken to indicate that the bicuspid valve was

congenital and the stenosis not rheumatic. In a further 7 the original authors considered thatthe bicuspid valve was congenital, while only one was proved on microscopy to have arisen fromfusion of two of three cusps.

It is concluded that Peacock's suggestion that degeneration in a congenital bicuspid valvecan lead to aortic stenosis is correct. The frequency of congenital bicuspid valve in association withcoarctation, its abnormal structure, and the higher pressures to which in coarctation the aortic valveis exposed, are considered to be the factors responsible for the development of aortic stenosis withcoarctation.

It is suggested that the importance of degeneration of the congenital bicuspid valve as a causeof isolated calcific aortic stenosis has not received sufficient attention. This is attributed to thedifficulty of morphological interpretation when the deformity and calcification are far advanced.

The clinical features of the syndrome are briefly discussed.

We are grateful to Dr. J. F. Goodwin for putting at our disposal the records of Cases 2 and 4, and to Dr. JeanBret of Lyons for the details of Case 3; to Dr. Raymond Daley for the specimen shown in Fig. 2; to Dr. A. C. Dorn-horst for helpful criticism; to Miss Joan Dewe for the illustrations and to Mr. A. L. Wooding for their reproduction.

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